Interstitial Lung Disease in Systemic Sclerosis

Wells, Athol U.; Margaritopoulos, George; Αντωνίου, Κατερίνα; Denton, Chris

doi:10.1055/s-0034-1371541

Cited by 47 publications

(11 citation statements)

References 96 publications

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“…As somewhat effective therapies for other manifestations of SSc (e.g., renal, pulmonary arterial hypertension, and articular) have emerged [ 27 , 28 ], the morbidity and mortality of ILD have become increasingly apparent [ 29 – 31 ]. Traditionally, the severity of SSc-ILD is defined by the degree of ventilatory restriction in conjunction with the magnitude of diffusion impairment.…”

Section: Discussionmentioning

confidence: 99%

Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis–associated interstitial lung disease trials

Khanna¹,

Nagaraja²,

Tseng³

et al. 2015

Arthritis Res Ther

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BackgroundThe extent of lung involvement visualized by high-resolution computed tomography (HRCT) is a predictor of decline in forced vital capacity (FVC) in scleroderma–interstitial lung disease. Our objective was to evaluate the performance of three different HRCT-defined staging systems in the Scleroderma Lung Study I (SLS I) over a 1-year period.MethodsWe assessed two visual semiquantitative scores: the maximum fibrosis score (MaxFib, the fibrosis score in the zone of maximal lung involvement) and visual assessment of total lung involvement (TLI) as proposed by Goh and Wells. In addition, we evaluated the computer-aided diagnosis and calculated the quantitative percentage with fibrosis (QLF) and TLI.ResultsThe mean duration of the disease was 3.2 years, and the mean FVC was 67.7 %. Regardless of the staging system used, a greater degree of fibrosis/TLI on HRCT scans was associated with a greater decline in FVC in the placebo group. Using the MaxFib and QLF, the mean absolute changes in FVC from baseline were 0.1 % and −1.4 %, respectively, in <25 % lung involvement vs. a change of −6.2 % and −6.9 %, respectively, with >25 % involvement (negative score denotes worsening in FVC). Conversely, cyclophosphamide was able to stabilize decline in FVC in subjects with greater degree of involvement detected by HRCT. Using the visual MaxFib and QLF, the mean absolute improvements in FVC were 1.2 and 1.1, respectively, with >25 % involvement.ConclusionsHRCT-defined lung involvement was a predictor of decline in FVC in SLS I. The choice of staging system for cohort enrichment in a clinical trial depends on feasibility.Trial registrationClinicalTrials.gov identifier: NCT00004563 (Scleroderma Lung Study I)ISRCTN15982171. Registered 19 Aug 2015.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0872-2) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis–associated interstitial lung disease trials

Khanna¹,

Nagaraja²,

Tseng³

et al. 2015

Arthritis Res Ther

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“…The clinical course of PH and ILD in SSc is highly variable ranging from slowly evolving cases that may respond to targeted therapy to more progressive forms that may result in right heart failure or pulmonary fibrosis with respiratory failure and death [ 7 , 8 ]. The pathological processes leading to SSc-PH and SSc-ILD are not well understood, but they most likely involve complex mechanisms driven by inappropriate immune activation and enhanced fibrogenesis in the lungs, but the molecules that drives these interacting processes are not fully understood and hamper targeted therapeutic intervention [ 2 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Augmented concentrations of CX3CL1 are associated with interstitial lung disease in systemic sclerosis

et al. 2018

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BackgroundDysregulation of Fractalkine (CX3CL1) and its receptor CX3CR1 has been linked to the pathobiology of chronic inflammatory conditions. We explored CX3CL1 in systemic sclerosis (SSc) related progressive interstitial lung disease (ILD) and pulmonary hypertension (PH) in two different but complementary sources of biomaterial.MethodsWe collected lung tissue at the time of lung transplantation at UCLA from SSc-ILD patients (n = 12) and healthy donors (n = 12); and serum samples from the prospective Oslo University Hospital SSc cohort (n = 292) and healthy donors (n = 100). CX3CL1 was measured by ELISA. Cellular sources of CX3CL1/CX3CR1 in lung tissues were determined by immunohistochemistry and immunofluorescence. ILD progression and new onset PH endpoints were analysed.ResultsCX3CL1 concentrations were increased in SSc in lung tissue as well as in sera. In the UCLA cohort, CX3CL1 was highly correlated with DLCO. In the SSc-ILD lungs, CX3CL1 was identified in reactive type II pneumocytes and airway epithelial cells. CX3CR1 stained infiltrating interstitial mononuclear cells, especially plasma cells. In the Oslo cohort, CX3CL1 correlated with anti-Topoisomerase-I-antibody and lung fibrosis. CX3CL1 was associated with ILD progression in multivariable regression analysis but not PH.ConclusionCX3CL1 is associated with progressive SSc-ILD but not SSc-PH. The CX3CR1/CX3CL1-biological axis may be involved in recruiting antibody secreting plasma cells to SSc lungs, thereby contributing to the immune-mediated pathobiology of SSc-ILD.

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“…29,49 The most prevalent pattern of SSc-ILD is non-specific interstitial pneumonia (NSIP), seen in up to 78% of cases, followed by usual interstitial pneumonia (UIP) in 25–40% of cases. 53,54 …”

Section: Systemic Sclerosismentioning

confidence: 99%

Corticosteroids in Myositis and Scleroderma

Postolova

Chen

Chung

2016

Rheumatic Disease Clinics of North America

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Synopsis Idiopathic inflammatory myopathies (IIM) involve inflammation of the muscles and are classified based on the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into four categories: dermatomyositis, polymyositis, inclusion body myositis, and immune mediated necrotizing myopathy. The term “scleroderma” refers to fibrosis of the skin. Localized scleroderma (morphea) is skin-limited, while systemic sclerosis (SSc) is associated with vascular and internal organ involvement. Although there is a paucity of randomized clinical trials, treatment with systemic corticosteroids (CS) is the standard of care for IIM with muscle and organ involvement. The extra-cutaneous features of systemic sclerosis are frequently treated with CS, however high doses have been associated with scleroderma renal crisis in high-risk patients. CS monotherapy is neither recommended for the cutaneous manifestations of dermatomyositis nor scleroderma. While CS can be effective first line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering.

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Interstitial Lung Disease in Systemic Sclerosis

Cited by 47 publications

References 96 publications

Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis–associated interstitial lung disease trials

Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis–associated interstitial lung disease trials

Augmented concentrations of CX3CL1 are associated with interstitial lung disease in systemic sclerosis

Corticosteroids in Myositis and Scleroderma

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